Robert Justo

Redefining Expectations of Long-Term Survival After the Fontan Procedure Twenty-Five Years of Follow-Up From the Entire Population of Australia and New Zealand

Background— The life expectancy of patients undergoing a Fontan procedure is unknown. Methods and Results— Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975–1995), 271 lateral tunnels (1988–2006), and 532 extracardiac conduits (1997–2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%–93%) for AP and 97% (95% confidence interval [CI], 94%–99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%–82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P<0.001; 95% CI, 2.4–16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%–76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P<0.001; 95% CI, 2.0–7.1). Ten-year freedom from failure was 79% (95% CI, 61%–89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%–95%) for other morphologies. Conclusions— The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure.

Neurodevelopmental Outcomes After Cardiac Surgery in Infancy

BACKGROUND: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD). METHODS: We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Development, second edition, after cardiac surgery between 1996 and 2009. The primary outcome was Psychomotor Development Index (PDI), and the secondary outcome was Mental Development Index (MDI). RESULTS: Among 1770 subjects from 22 institutions, assessed at age 14.5 ± 3.7 months, PDIs and MDIs (77.6 ± 18.8 and 88.2 ± 16.7, respectively) were lower than normative means (each P < .001). Later calendar year of birth was associated with an increased proportion of high-risk infants (complexity of CHD and prevalence of genetic/extracardiac anomalies). After adjustment for center and type of CHD, later year of birth was not significantly associated with better PDI or MDI. Risk factors for lower PDI were lower birth weight, white race, and presence of a genetic/extracardiac anomaly (all P ≤ .01). After adjustment for these factors, PDIs improved over time (0.39 points/year, 95% confidence interval 0.01 to 0.78; P = .045). Risk factors for lower MDI were lower birth weight, male gender, less maternal education, and presence of a genetic/extracardiac anomaly (all P < .001). After adjustment for these factors, MDIs improved over time (0.38 points/year, 95% confidence interval 0.05 to 0.71; P = .02). CONCLUSIONS: Early neurodevelopmental outcomes for survivors of cardiac surgery in infancy have improved modestly over time, but only after adjustment for innate patient risk factors. As more high-risk CHD infants undergo cardiac surgery and survive, a growing population will require significant societal resources.

Transcatheter perforation of the right ventricular outflow tract as initial therapy for pulmonary valve atresia and intact ventricular septum in the newborn

The clinical impact of transcatheter perforation and dilatation of the right ventricular outflow tract in neonates with pulmonary atresia and intact ventricular septum was reviewed. Between April 1992 and December 1994, 8 neonates underwent transcatheter perforation of the right ventricular outflow tract. Radiofrequency energy was employed in 6 patients and wire perforation in 2 patients. Mean patient age at intervention was 1.9 +/- 0.6 days and weight 3.4 +/- 0.5 kg. Median tricuspid valve annulus was 10.9 mm (range: 4.0-13.0 mm) and Z-value -0.85 (range: -4.5-1.0). The mean right ventricular systolic pressure fell from 117 +/- 16 to 55 +/- 15 mm Hg (P < 0.0001), and the right ventricular to aortic pressure ratio decreased from 1.81 +/- 0.33 to 0.82 +/- 0.28 (P < 0.0001). The arterial duct was patent in all. No acute complications occurred. Aortopulmonary shunts were performed in 7 patients at a median 6 days (range: 3-23 days) following catheterization. One patient developed sepsis and died after surgical resection of infected tissue, while a second patient died of a blocked aortopulmonary shunt 17 months following discharge. Median follow-up for the 6 surviving patients was 8 months (range: 4-32 months). One patient has achieved and a second is awaiting biventricular repair. Transcatheter perforation appears to be a promising form of therapy in selected patients with pulmonary atresia, and potentially facilitates algorithms leading to a biventricular repair.

Aortic valve regurgitation after surgical versus percutaneous balloon valvotomy for congenital aortic valve stenosis

To compare characteristics of aortic regurgitation (AR), the results of 213 procedures (110 balloon aortic valvotomies [BAV] and 103 surgical aortic valvotomies [SAV]) for treatment of congenital aortic valve stenosis were reviewed. These procedures were performed in 187 patients from June 1981 to September 1993. Echocardiograms recorded immediately before, within 6 months afterward, and at latest follow-up were compared. Color Doppler was used to assess the degree of AR and was quantified as the ratio of the regurgitant jet width to valve annulus, the jet width ratio. Whereas BAV patients were older (median age 5.7 years vs 3 months; p = 0.0001), there was no significant difference in median follow-up interval (3.1 years [range 0.5 to 7.2] for BAV vs 3.6 years [range 0.6 to 10.4] for SAV; p = 0.44). The mean balloon-to-annulus ratio for BAV was 0.99 +/- 0.09. An open valvotomy was performed in 83% of surgical cases. Acute systolic gradient reduction and subsequent increase at late follow-up was similar for both groups. Acutely, the mean jet width ratio increased similarly (p = 0.84) for BAV (+9 +/- 15%; p = 0.0001) and SAV (+9 +/- 12%; p = 0.0003) and was not related to age at procedure. At late follow-up, mean jet width ratio further increased significantly in both groups, although there was no difference (p = 0.17) in amount of progression (BAV +10 +/- 12%; p = 0.0001, SAV +15 +/- 13%; p = 0.0002). Thus, BAV and SAV produce AR of similar severity with similar rates of progression.

Long-Term Outcomes of Dilated Cardiomyopathy Diagnosed During Childhood: Results from a National Population-Based Study of Childhood Cardiomyopathy

Background— Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy. Methods and Results— The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings. The primary study end point included time to the combined outcome of death or cardiac transplantation. There were 175 patients 0 to <10 years of age at the time of diagnosis. Survival free from death or transplantation was 74% (95% confidence interval, 67–80) 1 year after diagnosis, 62% (95% confidence interval, 55–69) at 10 years, and 56% (95% confidence interval, 46–65) at 20 years. In multivariable analysis, age at diagnosis <4 weeks or >5 years, familial cardiomyopathy, and lower baseline left ventricular fractional shortening Z score were associated with increased risk of death or transplantation, as was lower left ventricular fractional shortening Z score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline left ventricular fractional shortening Z score, higher left ventricular fractional shortening Z score during follow-up, and greater improvement in left ventricular fractional shortening Z score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At the latest follow-up, 100 of 104 of survivors (96%) were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy. Conclusions— Death or transplantation occurred in 26% of patients with childhood dilated cardiomyopathy within 1 year of diagnosis and ~1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy, and severity of left ventricular dysfunction. The majority of surviving subjects are well and free of cardiac medication.Background— Existing studies of childhood dilated cardiomyopathy deal mainly with early survival. This population-based study examines long-term outcomes for children with dilated cardiomyopathy. Methods and Results— The diagnosis of dilated cardiomyopathy was based on clinical, echocardiographic, and pathological findings. The primary study end point included time to the combined outcome of death or cardiac transplantation. There were 175 patients 0 to 5 years, familial cardiomyopathy, and lower baseline left ventricular fractional shortening Z score were associated with increased risk of death or transplantation, as was lower left ventricular fractional shortening Z score during follow-up. At 15 years after diagnosis, echocardiographic normalization had occurred in 69% of surviving study subjects. Normalization was related to higher baseline left ventricular fractional shortening Z score, higher left ventricular fractional shortening Z score during follow-up, and greater improvement in left ventricular fractional shortening Z score. Children with lymphocytic myocarditis had better survival and a higher rate of echocardiographic normalization. At the latest follow-up, 100 of 104 of survivors (96%) were free of cardiac symptoms, and 83 (80%) were no longer receiving pharmacotherapy. Conclusions— Death or transplantation occurred in 26% of patients with childhood dilated cardiomyopathy within 1 year of diagnosis and ~1% per year thereafter. Risk factors for death or transplantation include age at diagnosis, familial cardiomyopathy, and severity of left ventricular dysfunction. The majority of surviving subjects are well and free of cardiac medication. # Clinical Perspective {#article-title-45}

Clinical impact of transcatheter closure of secundum atrial septal defects with the double umbrella device

The clinical impact of transcatheter closure of the isolated secundum atrial septal defect was reviewed. Closure by echocardiographic evaluation was 23 +/- 14% at 6 months, 49 +/- 16% at 2 years, and 64 +/- 16% at 4 years, and right ventricular end-diastolic dimensions in patients without residual shunts did not differ significantly from those with residual shunts.

Outcomes after balloon dilation of congenital aortic stenosis in children and adolescents

OBJECTIVES To determine the long-term outcomes and risk factors for, reintervention after balloon dilation of congenital aortic stenosis in children aged 6 months or older. BACKGROUND Although balloon dilation of congenital aortic stenosis has become a primary therapeutic strategy, few data are available regarding long-term outcomes. METHODS We carried out a retrospective review of 87 children who had undergone balloon dilation of the aortic valve at median age of 6.9 years. RESULTS The procedure was completed in 98% of the children, with an average reduction in the gradient across the valve of 64 +/- 28%, and without mortality. Of the children, 76 had been followed for a mean of 6.3 +/- 4.2 years. Reintervention on the aortic valve was required in 32 children, with 12 undergoing reintervention within 6 months, with 1 death. Another patient had died over the period of follow-up due to a non-cardiac event. Estimated freedom from reintervention was 86% at 1 year, 67% at 5 years, and 46% at 12 years. Parametric modeling of the hazard function showed a brief early phase of increased risk, superimposed on an ongoing constant risk. The only incremental risk factor for the early phase was a residual gradient immediately subsequent to the procedure greater than 30 mmHg. Incremental risk factors for the constant phase included the presence of symmetric valvar opening, and greater than moderate regurgitation immediately after dilation. CONCLUSION Long-term survival was excellent, albeit that the need for further reintervention was high due to the palliative nature of the procedure.

The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes

OBJECTIVES To identify factors associated with hospital and long-term outcomes in a binational cohort of extracardiac conduit (ECC) Fontan recipients. METHODS All patients who underwent an ECC Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified, and perioperative, follow-up, echocardiographic and reintervention data collected. Risk factors for early and late mortality, failure and adverse outcomes were analysed. RESULTS A total of 570 patients were identified, and late follow-up was available in 529 patients. The mean follow-up was 6.7 years (standard deviation: 3.5) and completeness of the follow-up was 98%. There were seven hospital mortalities (1%) and 21 patients (4%) experienced early failure (death, Fontan takedown/revision or mechanical circulatory support). Prolonged length of stay occurred in 10% (57 patients), and prolonged effusions in 9% (51 patients). Overall survival at 14 years was 96% (95% confidence interval [CI]: 93-98%), and late survival for patients discharged with intact Fontan was 98% (95% CI: 94-99%). The rates of late failure (late death, transplantation, takedown, New York Heart Association class III/IV or protein-losing enteropathy) and adverse events (late failure, reoperation, percutaneous intervention, pacemaker, thromboembolic event or supraventricular tachycardia) per 100 patient-years were 0.8 and 3.8, and their 14-year freedoms were 83% (95% CI: 70-91%) and 53% (95% CI: 41-64%), respectively. After adjustment for confounders, hypoplastic left heart syndrome (HLHS) was strongly associated with prolonged effusions (OR: 2.9, 95% CI: 1.4-5.9), late failure (hazard ratio [HR]: 2.8, 95% CI: 1.1-7.5) and adverse events (HR: 3.6, 95% CI: 1.3-7.5). CONCLUSIONS The extracardiac Fontan procedure provides excellent survival into the second decade of life, but half of patients will suffer a late adverse event by 14 years. Patients with HLHS are at higher risk of late adverse events than other morphological groups, but their survival is still excellent.

The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry

The Fontan procedure is the final in a series of staged palliations for single‐ventricle congenital heart disease, which encompasses rare and heterogeneous cardiac lesions. It represents an unusual and novel physiological state characterised by absence of a subpulmonary ventricle.

Postoperative junctional ectopic tachycardia (JET)

Bei der postoperativen junktionalen ektopen Tachykardie (JET) handelt es sich um eine potentiell lebensbedrohliche tachykarde Herzrhythmusstörung, die meistens nach operativer Korrektur angeborener Herzfehler auftritt. Die Arrhythmie selbst sowie ihre Behandlung hat bedeutsame klinische Auswirkung auf den postoperativen Verlauf der Patienten. Im Allgemeinen handelt es sich bei der JET um eine selbst-limitierende Tachykardie, die innerhalb einer Woche sistiert. Allerdings tritt die Tachykardie üblicherweise innerhalb der ersten 24 bis 48 Stunden postoperativ auf, zu einem Zeitpunkt, wenn die systolische und diastolische Funktion am meisten beeinträchtigt ist. Die schnelle Herzfrequenz führt zu einer akuten weiteren Verschlechterung des Herzzeitvolumens und bedarf somit einer raschen effektiven Therapie. Die Diagnose einer JET wird durch das typische EKG mit schmalen QRS-Komplexen, einer Frequenz von 170 bis 260/min und einer AV-Dissoziation gestellt. Zur Therapie der JET wurden verschiedene Therapiestrategien eingesetzt. Eine erfolgreiche Therapie ist hierbei definiert als eine Reduktion der Ventrikelfrequenz unter 140–150/min und damit der Möglichkeit, erfolgreich eine Vorhofstimulation zur Verbesserung des Herzminutenvolumens zu erzielen. Eine optimale Therapie stellt das Erreichen eines Sinusrhythmus dar. In vielen Einrichtungen wird eine postoperative JET anhand eines speziellen Therapieprotokolls behandelt. Die Therapiestrategien beinhalten allgemeine supportive Maßnahmen zur Stabilisierung der Patienten, eine spezifische antiarrhythmische Therapie, verschiedene Formen der Schrittmachertherapie sowie die Induktion einer moderaten Hypothermie. Durch die Einführung von Amiodarone in hoher Dosis kann man heutzutage eine zufriedenstellende Reduktion der Herzfrequenz zu erzielen, die eine atriale Schrittmacherstimulation ermöglicht. Eine Katheter-Ablation des His-Bündels oder gar chirurgische Interventionen ist nur in seltenen Ausnahmefällen notwendig. In dem vorliegenden Artikel wird die Literatur zur postoperativen JET zusammengefasst und analysiert sowie ein in unserer Abteilung bewährter Algorhythmus zur Therapie der postoperativen JET dargestellt. Postoperative junctional ectopic tachycardia (JET) is a potential life-threatening tachycardia that mainly occurs after surgical correction of congenital heart defects. The arrhythmia itself or the related treatment has significant clinical impact on the postoperative course and intensive care stay. In general, JET is a self-limiting disorder that usually resolves within one week. However, JET occurs usually within the first 24 to 48 hours after corrective surgery, when systolic and diastolic function of the heart is impaired. Thus, the rapid heart rate leads to an acute further deterioration of cardiac output that requires adequate treatment. The diagnosis of JET is made by the typical ECG-appearance with narrow QRS-configuration at a rate of 170 to 260 bpm and AV-dissociation. A variety of different therapeutic strategies have been tested in postoperative and congenital/spontaneous JET. Treatment success is usually defined as a stable decrease in the ventricular rate below 140–150/min, the possibility of atrial pacing and thereby the improvement of cardiac output. Optimal success is the reinstitution of sinus rhythm. Many of the treatment strategies reported are based on specific institutional treatment protocols. These include conventional supportive treatment, specific medical antiarrhythmic therapy, specific forms of pacing and surface cooling. Today, the administration of high doses of amiodarone usually leads to adequate control of the rate and enables pacing. Surgical intervention or catheter ablation of the HIS-bundle is rarely necessary. This article reviews the literature about JET over the past years and offers a specific treatment protocol.